ATI RN
The Hematologic System ATI Questions
Question 1 of 5
A 4-year-old boy is pale with intermittent jaundice and splenomegaly. Laboratory results are as follows: RBC 4.85 M/mcL (N); Hgb 8.6 g/dL (L); Hct 25.8% (L); MCV 81.6 (N); MCHC 38% (H); RDW 20% (H); Retic 7% (H). What are the two best tests to distinguish autoimmune hemolytic anemia from hereditary spherocytosis?
Correct Answer: E
Rationale: As the correct answer is not provided, I'll provide a step-by-step rationale for each choice: A: Free erythrocyte protoporphyrin and IgG levels are not specific tests for distinguishing between autoimmune hemolytic anemia and hereditary spherocytosis. B: Hemoglobin electrophoresis and direct antiglobulin test (DAT) can help differentiate between these two conditions as autoimmune hemolytic anemia is associated with a positive DAT, while hereditary spherocytosis typically has a negative DAT. C: Lactate dehydrogenase (LDH) and modified Russell viper venom test are not specific tests for distinguishing between autoimmune hemolytic anemia and hereditary spherocytosis. D: Red cell distribution width (RDW) and mean corpuscular hemoglobin concentration (MCHC) are not typically used to distinguish between autoimmune hemolytic anemia and hereditary spherocytosis. Therefore, the best tests
Question 2 of 5
An 18-year old male patient presents with bruising, fatigue, and diffuse extremity pain. He is noted to be tachypneic and hypoxic and has a diffuse interstitial infiltrate on chest x-ray. CBC reveals a WBC count of 285,000/mm3 (85% myeloblasts, with monocytic morphology), hemoglobin of 7.9 g/dL, and platelet count of 36,000/mm3. What is the most likely cause of the infiltrate and respiratory symptoms and the most appropriate initial treatment?
Correct Answer: B
Rationale: The correct answer is B. The patient's presentation with tachypnea, hypoxia, and diffuse interstitial infiltrate on chest x-ray suggests leukostasis syndrome due to hyperleukocytosis. The extremely high WBC count of 285,000/mm3 with myeloblasts indicates acute myeloid leukemia. Leukapheresis or manual exchange transfusion is needed to rapidly reduce the number of leukemic blasts in circulation to prevent complications like tissue hypoxia. Initiation of induction chemotherapy is essential for long-term management of AML. Choice A is incorrect because induction chemotherapy alone may not rapidly reduce the WBC count in cases of leukostasis. Choice C is incorrect as the patient's clinical scenario is not consistent with COVID-19 infection, and convalescent plasma is not indicated for leukostasis. Choice D is incorrect as the patient's symptoms are not typical for pneumococcal pneumonia, and vancomycin is not the initial
Question 3 of 5
You are consulting on a 10-year-old male with severe persistent neutropenia, a history of recurrent infections, and warts. The rest of the peripheral blood count is normal. His mother also has neutropenia. Bone marrow examination shows a hypercellular marrow and retained myeloid cells with vacuolated cytoplasm. There are no abnormalities in the red cells or platelet precursors. Cytogenetics are 46XY. You start granulocyte colony stimulating factor therapy and the neutrophil count increases. A mutation in which of the following genes is most likely to have caused this familial inherited bone marrow failure syndrome?
Correct Answer: A
Rationale: The correct answer is A: CXCR4. In this case, the familial inherited bone marrow failure syndrome with severe neutropenia, recurrent infections, and warts suggests WHIM syndrome. WHIM syndrome is caused by a gain-of-function mutation in the CXCR4 gene, leading to impaired neutrophil trafficking. The symptoms and bone marrow findings in this patient align with WHIM syndrome. Choice B (ELANE) is associated with cyclic neutropenia, not WHIM syndrome. Choice C (GATA 2) is linked to familial myelodysplastic syndromes and acute myeloid leukemia, not WHIM syndrome. Choice D (Mitochondrial DNA) is not associated with WHIM syndrome. Therefore, the mutation in CXCR4 is the most likely cause of the familial inherited bone marrow failure syndrome in this patient.
Question 4 of 5
Several gene mutations have been associated with juvenile myelomonocytic leukemia (JMML), and they may or may not have prognostic implications. A gene expression–based classification system has been found to be an independent predictor of clinical outcome in these patients. What is the disease signature that predicts a poor outcome?
Correct Answer: B
Rationale: The correct answer is B: Acute myeloid leukemia-like. This is because JMML shares similarities with acute myeloid leukemia in terms of aggressive progression and poor outcomes. Children with JMML who exhibit an acute myeloid leukemia-like gene expression signature have been shown to have a worse prognosis compared to those with other gene expression profiles. The other choices (A, C, D) are incorrect because tyrosine kinase inhibitors are not directly related to predicting clinical outcomes in JMML, chronic myeloid leukemia-like gene expression profile does not necessarily predict poor outcomes in JMML, and BRAF pathway abnormalities are not specifically associated with predicting poor outcomes in JMML.
Question 5 of 5
You have been asked to see a 15-year-old girl who is being referred for evaluation of an ovarian mass. Her history is also significant for secondary amenorrhea, and physical examination shows signs of virilization. As you review her family history, what syndrome will you consider?
Correct Answer: B
Rationale: The correct answer is B: DICER-1 syndrome. This syndrome is associated with ovarian tumors, secondary amenorrhea, and signs of virilization. DICER-1 gene mutations can lead to the development of Sertoli-Leydig cell tumors, which can present with these symptoms in adolescent girls. Li-Fraumeni syndrome (A) is characterized by a predisposition to various cancers but not specifically ovarian tumors. Turner syndrome (C) is associated with ovarian dysgenesis leading to primary amenorrhea, not secondary amenorrhea and virilization. Beckwith-Wiedemann syndrome (D) is a genetic overgrowth syndrome with a risk of embryonal tumors but not typically ovarian masses with virilization.